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MOTOR-NERVE DISEASE MAY NOT BE LOU GEHRIG'S

Q: I have Lou Gehrig's disease. I cannot speak at all anymore and it is very hard for me to eat, as my tongue does not move at all. Also, to swallow is very hard, so I have to grind all my food and thicken all my liquid.

I have a very serious problem with drooling and doctors have found nothing to help it. It runs out steady. Do you know of anything that could be done? The rest of my body is fine. I walk two miles every day.

-- C.S.
A: I'm so sorry to hear of your condition. Amyotrophic lateral sclerosis (ALS, Lou Gehrig's disease) and other related motor-neuron diseases are often devastating physically and emotionally.

ALS is one of a group of disorders that have progressive deterioration of the motor nerves, which stimulate muscle activity. Sensory nerves are not affected by these disorders. Without proper stimulation from nerves, muscles weaken, shrink (atrophy) and can even become paralyzed.

Each of these disorders affects different nerves and therefore different muscle groups. The disorders are more common in men and symptoms usually begin between 30 and 60 years of age.

ALS or one of the other disorders of muscle stimulation would be suspected when a person experiences progressive muscle weakness without loss of sensation, e.g., touch, heat or cold. Further laboratory studies that measure the electrical activity of muscles and nerves are used to help confirm the diagnosis. Muscle biopsies also may be recommended.

But in ALS, nerves to the head and nerves to the limbs are affected simultaneously. From what you've said, your limbs are not affected, so my guess is that you have one of the other types of motor-neuron diseases. And because your tongue doesn't move at all, the disease called progressive bulbar palsy is more likely.

Sadly, the cause of bulbar palsy, ALS or any of the other motor-neuron diseases is unknown and there is no cure. The drug riluzole may slow progression, at least in ALS, but there is no other specific treatment to stop the deterioration of the nerves.

Research in the use of other medications is in progress. For example, a recent study showed that the amino acid creatine is helpful in mice with a similar condition. Some researchers are experimenting with substances that promote the growth of nerves.

Otherwise, treatment is limited to controlling the severity of symptoms, e.g., with physical therapy and supportive care. Braces and walkers are helpful for those with mobility problems.

Physical therapy is suggested to prevent muscles from tightening (contractures). The drugs diazepam or baclofen may help to reduce muscle spasms and relieve muscle cramps.

Anticholinergic drugs that dry mucous membranes, such as trihexyphenidyl, amitripyline or atropine, may help with your drooling.

Although treatment today is to help specific symptoms of the motor-neuron diseases and there is little difference from one to the other, knowing which one you have is critical in projecting what problems you may develop and how to best direct future treatment.

Based on what you described as your symptoms, I strongly suggest that you revisit your doctor and consider review by a neurologist to make sure what your diagnosis is. Also, follow medical news, especially on the Internet, for newdevelopments.

Update on sugar measurements: As early as this April, you may be able to buy a medical instrument worn on the wrist like a watch that accurately measures the level of sugar (glucose) in your blood.

You simply place the device, called a "Glucowatch," on the skin -- without puncturing the skin -- and it will continuously track your glucose levels. Obviously this provides a great deal benefit because of comfort.

But it may also provide valuable information about the subtle changes in one's glucose levels, not only for diabetics but also for people concerned about the effects of sugar levels and insulin on cardiovascular disease.

Dr. Allen Douma welcomes questions from readers. Although he cannot respond to each one individually, he will answer those of general interest in his column. Write to Dr. Douma in care of Tribune Media Services, 435 N. Michigan Ave., Suite 1400, Chicago, Ill. 60611. His e-mail address is DRFamily@aol.com.

This column is for informational and educational purposes only. It is not intended to provide medical advice or take the place of consultation with a doctor or other health-care provider.

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